肺淋巴管平滑肌瘤病2例诊疗分析

Diagnosis Analysis on 2 Cases of Pulmonary Lymphangioleiomyomatosis

目的:提高对肺淋巴管平滑肌瘤病(PLAM)的认识,减少误诊。方法:对2例PLAM的临床资料并结合文献进行分析。结果:PLAM为一种弥漫性肺间质疾病,临床表现呼吸困难、咯血、气胸、乳糜胸等,极易被误诊。胸部HRCT具有特征性改变,显示两侧对称弥漫性广泛囊状影。结论:对于年轻育龄期女性反复出现气胸、乳糜胸,胸部HRCT提示囊状改变,需考虑PLAM,预后差,无特效治疗。

Objective： Improving the cognition on pulmonary lymphangioleiomyomatosis and reduce the misdiagnosis. Methods： Making an analysis on the clinical data as well as documents of the two pulmonary lymphangioleiomyomatosis. Results： Pulmonary lymphangioleiomyomatosis is a diffuse pulmonary interstitial disease. Its clinical symptoms are anhelation, hemoptysis, pneumothorax, chylothorax, etc. and could be misdiagnosed very easily. The thorax HRCT shows a characteristic change with symmetric diffuse broad cystic shadow on both sides. Conclusion： If pneumothorax, chylothorax occur repeatedly to young females in child-bearing period and thorax HRCT shows a cystic change, pulmonary lymphangioleiomyomatosis should be taken into consideration, which is of poor prognosis, no specific treatment.